Eisenmenger syndrome

Eisenmenger syndrome (ES) is a form of pulmonary arterial hypertension (PAH) associated with unoperated congenital heart disease and is characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH.

Cyanosis

Bluish discoloration of the skin and mucosa due to poor circulation or inadequate oxygenation of arterial or capillary blood.


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(per page)
PMID (PMCID)
29201477
(5672606)
 OTHER
A Case of Advanced Glaucoma with Increased Episcleral Venous Pressure in a 17-Year-Old with Eisenmenger Syndrome.
Grech L, Mifsud A, Caruana M, Carbonaro F.
Case Rep Ophthalmol Med. 2017;2017:5808047.
Eisenmenger syndrome refers to reversal of shunt and central cyanosis due to pulmonary hypertension induced by congenital heart disease with a large systemic-to-pulmonary shunt.
23329721
 FEMALE Adult
Clubbing, cyanosis and a new diagnosis of atrial septal defect in a parturient: a straightforward diagnosis of Eisenmenger syndrome?
Butcher C, Plyman C, Hughes M, Walker F.
BMJ Case Rep. 2013;2013:.
Clubbing, cyanosis and a new diagnosis of atrial septal defect in a parturient: a straightforward diagnosis of Eisenmenger syndrome?
23329721
 FEMALE Adult
Clubbing, cyanosis and a new diagnosis of atrial septal defect in a parturient: a straightforward diagnosis of Eisenmenger syndrome?
Butcher C, Plyman C, Hughes M, Walker F.
BMJ Case Rep. 2013;2013:.
Eisenmenger syndrome was diagnosed with cyanosis due to shunt reversal at atrial level (right atrium to left), as a consequence of an elevated pulmonary vascular resistance from chronically increased pulmonary blood flow.
27122759
 OTHER
Late-Onset Cyanosis and Finger Clubbing in a 37-Year-Old Female with Ebstein's Anomaly and Atrial Septal Defect.
Chun KJ, Han DC, Park YH, Lee SK.
Acta Cardiol Sin. 2013;29(6):565-8.
Atrial septal defect; Cyanosis; Ebstein's anomaly; Eisenmenger syndrome.
18947936
 FEMALE Middle Aged
Pulmonary atherosclerosis associated with an atrial septal defect in old age: case report of an elderly autopsied patient.
Nascimento D, Nunes L, Oliveira F, Vencio E, Teixeira V, Reis M.
Pathol Res Pract. 2009;205(2):137-41.
This hypertension probably became intense to the point of reversing the direction of the shunt, from the right to the left, characterizing a probable Eisenmenger syndrome, responsible for the patient's dyspnea and cyanosis.
17822253
 FEMALE Middle Aged
[Two patients with Eisenmenger syndrome treated with novel agents that target vasodilation of the pulmonary capillary bed].
Hamaker ME, Duffels MG, Reichert CL, Cornel JH, Mulder BJ, Umans VA.
Ned Tijdschr Geneeskd. 2007;151(32):1795-9.
Two women with Eisenmenger syndrome, aged 63 and 45 years, presented with different symptoms: the first patient had peripheral oedema, proteinuria, progressive fatigue and cyanosis and the other had increasing dyspnoea and blue lips.
10386703
 FEMALE Child
Palliative arterial switch for complete transposition with ventricular septal defect.
Elizari A, Somerville J.
Cardiol Young. 1999;9(3):315-8.
She is married, has been counselled against pregnancy and has increasing cyanosis with the typical features of the Eisenmenger syndrome.